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01-06-2012 | INFLAMMATORY MUSCLE DISEASE (I LUNDBERG, SECTION EDITOR)

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment

Authors: Tsuneyo Mimori, Ran Nakashima, Yuji Hosono

Published in: Current Rheumatology Reports | Issue 3/2012

Abstract

Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis–ILD. Rituximab may become another candidate if these drugs are not effective.

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Title: Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment
Authors: Tsuneyo Mimori
Ran Nakashima
Yuji Hosono
Publication date: 01-06-2012
Publisher: Current Science Inc.
Published in: Current Rheumatology Reports / Issue 3/2012
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-012-0246-6

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